POS1372 THE 2021 EULAR AND ACR POINTS TO CONSIDER FOR DIAGNOSIS, MANAGEMENT AND MONITORING OF THE IL 1 MEDIATED AUTOINFLAMMATORY DISEASES: CAPS, TRAPS, MKD, AND DIRA
Authors: M. Romano, Z. S. Arici, D. Piskin, S. Alehashemi, D. Aletaha, K. Barron, S. Benseler, R. Berard, L. Broderick, F. Dedeoglu, M. Diebold, K. Durrant, P. Ferguson, D. Foell, J. Hausmann, O. Jones, D. Kastner, H. J. Lachmann, R. Laxer, D. Rivera, N. Ruperto, A. Simon, M. Twilt, J. Frenkel, H. Hoffman, A. De Jesus, J. Kuemmerle-Deschner, S. Özen, M. Gattorno, R. Goldbach-Mansky, E. Demirkaya
Journal: Annals of the Rheumatic Diseases (2022)

Background The Interleukin-1 (IL-1) mediated systemic autoinflammatory diseases (SAIDs), including the cryopyrin-associated periodic syndromes (CAPS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), and deficiency of the IL-1 receptor antagonist (DIRA) belong to a group of rare immunodysregulatory diseases that primarily present in early childhood with variable multiorgan involvement. When untreated, patients with severe clinical phenotypes have a poor prognosis, and diagnosis and management of these patients can be challenging. However, recently-approved treatments targeting the pro-inflammatory cytokine IL-1 have been life-changing and have significantly improved patient outcomes. Objectives We aimed to establish evidence-based recommendations on diagnosis, treatment, and monitoring to standardize the management of these patients. Methods A multinational, multidisciplinary task force consisting of physician experts including rheumatologists, patients or caregivers, and allied health care professionals was established. Evidence synthesis including systematic literature review and expert consensus (Delphi) via surveys were conducted. Consensus methodology was utilized to formulate and vote on statements to guide optimal patient care. Results The task force devised five overarching principles, 14 statements related to diagnosis, 10 on therapy, and 9 focused on long-term monitoring that were evidence and/or consensus-based for patients with IL-1 mediated diseases. An outline was developed for disease-specific monitoring of inflammation-induced organ damage progression and reported therapies of CAPS, TRAPS, MKD, and DIRA. Conclusion The 2021 EULAR/ACR points to consider provide state-of-the-art knowledge based on published data and expert opinion to guide diagnostic evaluation, treatment, and monitoring of patients with CAPS, TRAPS, MKD and DIRA, and inform the various stakeholders about optimized patient care to improve disease outcomes. References [1]Masters SL, Simon A, Aksentijevich I, et al. Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease (*). Annu Rev Immunol 2009;27:621-68. Disclosure of Interests micol romano: None declared, Zehra Serap Arici: None declared, David Piskin: None declared, Sara Alehashemi: None declared, Daniel Aletaha Speakers bureau: Lilly, Merck, Pfizer, Roche, Sandoz, Consultant of: Abbvie, Amgen, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Grant/research support from: Abbvie, Amgen, Lilly, Novartis, Roche, SoBi, Sanofi, Karyl Barron: None declared, Susa Benseler: None declared, Roberta Berard Consultant of: Sandoz and Roche, Lori Broderick Grant/research support from: Novartis and Regeneron, Fatma Dedeoglu Consultant of: Novartis, Michelle Diebold: None declared, Karen Durrant: None declared, Polly Ferguson Consultant of: Novartis, Grant/research support from: NIH, CARRA, Inc, Dirk Foell Speakers bureau: Novartis, Peer Voice and SoBi, Consultant of: Boehringer Ingelheim, Chugai-Roche, Merck, Novartis, SoBi, Grant/research support from: Novartis and SoBi, Jonathan Hausmann Consultant of: Novartis, Biogen and Pfizer, Grant/research support from: CARRA, SoBi, Olcay Jones: None declared, Daniel Kastner: None declared, Helen J. Lachmann: None declared, Ronald Laxer Consultant of: SoBi, Novartis, Sanofi, Dorelia Rivera: None declared, Nicolino Ruperto Speakers bureau: Eli-Lilly, GSK, Pfizer SoBi and UCB, Consultant of: Ablynx, Amgen, Astrazeneca-Medimmune, Aurinia, Bayer, Bristol Myers and Squib, Cambridge Healthcare research, Celgene, Domain therapeutic, Eli-Lilly, EMD Serono, GSK, Idorsia, Janssen, Novartis, SoBi, Pfizer and UCB, Anna Simon: None declared, Marinka Twilt: None declared, Joost Frenkel: None declared, Hal Hoffman Consultant of: Novartis, Regeneron, SoBi, Aclaris, Grant/research support from: Bristol-Meyer-Squib, Jecure, Takeda and Zomagen, Adriana de Jesus: None declared, Jasmin Kuemmerle-Deschner Speakers bureau: Novartis and SoBi, Consultant of: Novartis and SoBi, Grant/research support from: Novartis and SoBi, Seza Özen Speakers bureau: Novartis and SoBi, Marco Gattorno Speakers bureau: Novartis and SoBi, Grant/research support from: Novartis, Raphaela goldbach-mansky: None declared, Erkan Demirkaya Grant/research support from: Sobi